Treatment of Guillain-Barré Syndrome with IVIG
Intravenous immunoglobulin (IVIG) at 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) is the first-line treatment for GBS patients with significant disability and should be initiated as early as possible, preferably within 2 weeks of symptom onset. 1, 2
First-Line Treatment Protocol
IVIG is preferred over plasma exchange as first-line therapy because it is easier to administer, more widely available, has higher completion rates, and causes fewer adverse effects, despite being more expensive ($12,000-16,000 vs $4,500-5,000 for PE). 1, 3
Dosing and Administration
- Standard dose: 0.4 g/kg body weight daily for 5 consecutive days 1, 2
- Total cumulative dose: 2 g/kg 4, 3
- Timing: Most effective when started within 2 weeks of symptom onset 1
- Treatment indication: Initiate in patients with GBS disability score ≥3 (unable to walk unaided) 1
Efficacy Evidence
Moderate quality evidence from seven trials with 623 severely affected participants demonstrates that IVIG hastens recovery as much as plasma exchange, with no statistically significant difference in disability improvement after 4 weeks (mean difference 0.02 grade on a 7-point scale, 95% CI -0.20 to 0.25). 3 However, IVIG is significantly more likely to be completed than PE, making it the practical first choice. 3
Subtype-Specific Considerations
A critical caveat: IVIG efficacy varies by GBS subtype. In acute inflammatory demyelinating polyradiculoneuropathy (AIDP), IVIG results in significantly less poor recovery at 6 months (0.8% vs 6.6%, p=0.03), but in acute motor axonal neuropathy (AMAN), IVIG does not alter outcomes compared to natural course. 5 This suggests that treatment customization based on neurophysiological subtype may be warranted, though current guidelines do not yet incorporate this distinction.
When IVIG Alone May Be Insufficient
Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does NOT indicate treatment failure, as recovery can continue for more than 5 years. 1, 2 However, specific scenarios require additional intervention:
Treatment-Related Fluctuations (TRFs)
- Occur in 6-10% of patients within 2 months of initial improvement 1, 2
- Management: Repeat the full course of IVIG (0.4 g/kg/day × 5 days) 2
Pharmacokinetic Variability
A critical pitfall: Patients show large variation in IgG pharmacokinetics after standard dosing. Those with low serum IgG increase (ΔIgG) 2 weeks post-treatment recover significantly more slowly and are less likely to walk unaided at 6 months. 6 Consider measuring serum IgG levels 2 weeks post-treatment; patients with inadequate response may benefit from a second course or higher dosage. 6
Severe or Refractory Cases
For grade 3-4 events with inadequate response to IVIG alone, consider:
- Pulse corticosteroids (methylprednisolone 1 g/day for 5 days) along with IVIG 7
- Plasma exchange as alternative or sequential therapy 7, 1
- Note: Adding PE after IVIG does not confer significant extra benefit (mean grade improvement 0.2,95% CI -0.14 to 0.54) 3
Special Populations
Children
IVIG is strongly preferred over PE (0.4 g/kg/day × 5 days) due to better tolerability and fewer complications. 1, 2 Low quality evidence from three studies with 75 children shows IVIG significantly hastens recovery compared to supportive care alone (MD 1.42 grades, 95% CI 0.27 to 2.57). 3
Immune Checkpoint Inhibitor-Related GBS
Unlike idiopathic GBS where corticosteroids alone are not recommended, ICI-related GBS responds favorably to corticosteroids. 7 Management includes:
- Permanently discontinue the causative ICI 4
- First-line: Methylprednisolone 2-4 mg/kg/day with slow taper 7
- Consider concurrent IVIG or plasma exchange for severe cases 7, 4
Pregnant Women
Both IVIG and PE are not contraindicated, but IVIG is preferred due to fewer monitoring requirements. 2
Critical Monitoring: The "20/30/40 Rule"
Approximately 20% of GBS patients require mechanical ventilation. 1 Assess respiratory failure risk using:
- Vital capacity <20 ml/kg, OR
- Maximum inspiratory pressure <30 cmH₂O, OR
- Maximum expiratory pressure <40 cmH₂O 1, 2
Patients meeting these criteria are at high risk and require ICU-level monitoring. 2
Medications to AVOID
Absolutely avoid these agents as they worsen neuromuscular function:
Essential Supportive Care
Beyond immunotherapy, aggressive management of complications is mandatory:
- Pain management: Use gabapentin, pregabalin, or duloxetine for neuropathic pain (common in GBS) 4
- DVT prophylaxis due to immobility 1
- Pressure ulcer prevention through regular repositioning 1
- Constipation/ileus management (very common) 2, 4
- Prevention of hospital-acquired infections (pneumonia, UTIs) 1